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Definition

Complex regional pain syndrome (CRPS) is an uncommon, chronic condition that usually affects your arm or leg. Rarely, complex regional pain syndrome can affect other parts of your body.

Complex regional pain syndrome is marked by intense burning or aching pain. You may also experience swelling, skin discoloration, altered temperature, abnormal sweating and hypersensitivity in the affected area.

The cause of complex regional pain syndrome isn't clearly understood, though it often follows an illness or injury. Treatment for complex regional pain syndrome is most effective when started early. In such cases, dramatic improvement and even remission are possible.

Symptoms

The main symptom of complex regional pain syndrome is intense pain, which gets worse over time. Additional signs and symptoms include:

  • "Burning" pain in your arm, leg, hand or foot.
  • Skin sensitivity.
  • Changes in skin temperature, color and texture. At times your skin may be sweaty; at other times it may be cold. Skin color can range from white and mottled to red or blue. Skin may become tender, thin or shiny in the affected area.
  • Changes in hair and nail growth.
  • Joint stiffness, swelling and damage.
  • Muscle spasms, weakness and loss (atrophy).
  • Decreased ability to move the affected body part.

Symptoms may change over time and vary from person to person. Most commonly, swelling, redness, noticeable changes in temperature and hypersensitivity (particularly to cold and touch) occur first. Over time, the affected limb can become cold and pale and undergo skin and nail changes as well as muscle spasms and tightening. Once these changes occur, the condition is often irreversible.

When to see a doctor
If you experience constant, severe pain that affects a limb and makes touching or moving that limb seem intolerable, see your doctor to determine the cause. It's important to treat complex regional pain syndrome early.

Causes

Complex regional pain syndrome occurs in two types with similar signs and symptoms, but different causes:

  • Type 1. Previously known as reflex sympathetic dystrophy syndrome, this type occurs after an illness or injury that didn't directly damage the nerves in your affected limb. About 90 percent of people with complex regional pain syndrome have type 1.
  • Type 2. Once referred to as causalgia, this type follows a distinct nerve injury.

Many cases of complex regional pain syndrome occur after a forceful trauma to an arm or a leg, such as a gunshot wound or shrapnel blast. Other major and minor traumas — such as surgery, heart attacks, infections, fractures and even sprained ankles — also can lead to complex regional pain syndrome. It's not well understood why these injuries can trigger complex regional pain syndrome.

Complications

If complex regional pain syndrome isn't diagnosed and treated at an early stage, the disease may progress to more disabling signs and symptoms. These may include:

  • Muscle wasting (atrophy). If you avoid moving an arm or a leg because of pain or if you have trouble moving a limb because of stiffness, your skin and muscles may begin wasting.
  • Contracture. You may also experience tightening of your muscles. This may lead to a condition in which your hand and fingers or your foot and toes contract into a fixed position.

Complex regional pain syndrome occasionally may spread from its source to elsewhere in your body in these patterns:

  • Continuity type. The symptoms may migrate from the initial site of the pain — for example, from your hand to your shoulder, trunk and face.
  • Mirror-image type. The symptoms may spread from one limb to the opposite limb.
  • Independent type. Sometimes, the symptoms may leap to a distant part of your body.
Preparing for your appointment

To get the best medical care, take time to prepare for your appointment. If you suspect you have complex regional pain syndrome, write down any symptoms you're experiencing — including the severity and location of your pain, stiffness or sensitivity. It's also a good idea to write down any questions you have for your doctor.

What to expect from your doctor
Your doctor is likely to ask you a number of questions, as well. Being ready to answer them may reserve time to go over any points you want to spend more time on. For complex regional pain syndrome, your doctor may ask:

  • Have you had a recent accident, illness or injury, such as trauma to your limbs, a heart attack or an infection?
  • Have you had surgery recently?
  • When did you first begin experiencing pain or burning?
  • How long have you been experiencing your symptoms?
  • Is the pain occasional or continual?
  • Does anything seem to improve or worsen your symptoms?
  • Have you experienced similar symptoms after past injuries?
Tests and diagnosis

Diagnosis of complex regional pain syndrome is based on a physical exam and your medical history. There is no single test that can definitively diagnose complex regional pain syndrome, but the following procedures may provide important clues:

  • Bone scan. A radioactive substance injected into one of your veins permits viewing of your bones with a special camera. This procedure may show increased circulation to the joints in the affected area.
  • Sympathetic nervous system tests. These tests look for disturbances in your sympathetic nervous system. For example, thermography measures the skin temperature and blood flow of your affected and unaffected limbs. Other tests can measure the amount of sweat on both limbs. Dissimilar results can indicate complex regional pain syndrome.
  • X-rays. Loss of minerals from your bones may show up on an X-ray in later stages of the disease.
  • Magnetic resonance imaging (MRI). Images captured by an MRI device may show a number of tissue changes.
Treatments and drugs

Dramatic improvement and even remission of complex regional pain syndrome is possible if treatment begins within a few months of your first symptoms. Often, a combination of various therapies is necessary. Your doctor will tailor your treatment based on your specific case. Treatment options include:

Medications
Doctors use various medications to treat the symptoms of complex regional pain syndrome. Over-the-counter nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen (Advil, Motrin, others) and naproxen sodium (Aleve), may ease pain and inflammation. In some cases, doctors may recommend prescription medications. For example, antidepressants, such as amitriptyline, and anticonvulsants, such as gabapentin (Neurontin), are used to treat pain that originates from a damaged nerve (neuropathic pain). Corticosteroids, such as prednisone, may reduce inflammation.

Your doctor may suggest bone-loss medications, such as alendronate (Fosamax) and calcitonin (Miacalcin). Opioid medications may be another option. Taken in appropriate doses, they may provide acceptable control of pain. However, they may not be appropriate if you have a history of substance abuse or lung disease.

Some pain medications, such as COX-2 inhibitors (Celebrex), may increase your risk of heart attack and stroke. It's wise to discuss your individual risks with your doctor.

Therapies

  • Applying heat and cold. Applying cold may relieve swelling and sweating. If the affected area is cool, applying heat may offer relief.
  • Topical analgesics. Various creams are available that may reduce hypersensitivity, such as lidocaine or a combination of ketamine, clonidine and amitriptyline.
  • Physical therapy. Gentle, guided exercising of the affected limbs may improve range of motion and strength. The earlier the disease is diagnosed, the more effective exercises may be.
  • Sympathetic nerve-blocking medication. Injection of an anesthetic to block pain fibers in your affected nerves may relieve pain in some people.
  • Transcutaneous electrical nerve stimulation (TENS). Chronic pain is sometimes eased by applying electrical impulses to nerve endings.
  • Biofeedback. In some cases, learning biofeedback techniques may help. In biofeedback, you learn to become more aware of your body so that you can relax your body and relieve pain.
  • Spinal cord stimulation. Your doctor inserts tiny electrodes along your spinal cord. A small electrical current delivered to the spinal cord results in pain relief.
Coping and support

Living with a chronic, painful condition can be challenging, especially when — as is often the case with complex regional pain syndrome — your friends and family don't believe you could be feeling as much pain as you describe. Share information from reliable sources about complex regional pain syndrome with those close to you to help them understand what you're experiencing.

Take care of your physical and mental health by following these suggestions:

  • Maintain normal daily activities as best you can.
  • Pace yourself and be sure to get the rest that you need.
  • Stay connected with friends and family.
  • Continue to pursue hobbies that you enjoy and are able to do.

If complex regional pain syndrome makes it difficult for you to do things you enjoy, ask your doctor about ways to get around the obstacles.

Keep in mind that your physical health can directly affect your mental health. Denial, anger and frustration are common with chronic illnesses.

At times, you may need more tools to deal with your emotions. Professionals including therapists or behavioral psychologists may be able to help you put things in perspective. They can also teach you coping skills that may help you, including relaxation techniques.

Sometimes, joining a support group, where you can share experiences and feelings with other people, is a good approach. Ask your doctor what support groups are available in your community.

Prevention

The following measures may help you reduce the risk of contracting complex regional pain syndrome:

  • Taking vitamin C after a wrist fracture. Studies have shown that people who take daily vitamin C supplements after wrist fracture have a lower risk of complex regional pain syndrome compared with those who don't take vitamin C.
  • Early mobilization after a stroke. Some research suggests that people who get out of bed and walk around soon after a stroke (early mobilization) lower their risk of complex regional pain syndrome.

How you feel pain

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Updated: 03/31/2009


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